Idiopathic Pulmonary Arterial Hypertension

Definition

Primary pulmonary hypertension (PPH) is a rare disease. It is high blood pressure
in the blood vessels of the lungs.

A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.

Causes

The cause of primary pulmonary hypertension (PPH) is unknown. Several factors may contribute to the development of the disease, including:

Autoimmune diseases

Exposure to certain drugs or chemicals

Genetic defects

Risk Factors

Primary pulmonary hypertension (PPH) is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:

Liver cirrhosis

Portal hypertension

HIV infection

Family history of PPH

Use of appetite suppressants (diet pills)

Cocaine use

Symptoms

Initial symptoms of primary pulmonary hypertension (PPH) may be minor. They will get progressively worse. PPH may cause:

Shortness of breath (when you are active or at rest)

Abnormally rapid, deep breathing—hyperventilation

Fatigue

Progressive weakness

Fainting spells

Lightheadedness

Coughing up blood

Bluish tint to the lips and skin—cyanosis

Swelling of the legs and hands

Chest pain

Lack of appetite

Cold hands and feet

Low blood pressure

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis of primary pulmonary hypertension (PPH) may be delayed. It is hard to detect until symptoms worsen.

Treatment

There is no cure for primary pulmonary hypertension (PPH). Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best plan for you. Treatment options include the following:

Medication

Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include:

Calcium channel blockers

Prostacylins

Digoxin

Anticoagulants

Diuretics

Vasodilators

Endothelin receptor antagonists

Phosphodiesterase-5 inhibitors

Supplemental Oxygen

If breathing becomes difficult, oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.

Lung Transplant or Heart-Lung Transplant

Defective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of primary pulmonary hypertension (PPH) or when other treatment methods fail.

Prevention

There are no current guidelines for preventing primary pulmonary hypertension (PPH) because the cause is not known.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.